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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Anti-IgA antibodies in common variable immunodeficiency (CVID): diagnostic workup and therapeutic strategy.
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Anti-IgA antibodies in common variable immunodeficiency (CVID): diagnostic workup and therapeutic strategy.

机译：常见可变免疫缺陷症（CVID）中的抗IgA抗体：诊断检查和治疗策略。

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摘要

Common Variable Immunodeficiency (CVID) patients who are seropositive for anti-IgA antibodies have a predisposition for anaphylactoid reactions to intravenous immunoglobulin replacement therapy (IVIG). Among 88 CVID patients, we identified eight with IgG anti-IgA antibodies (9%). All eight completely lacked IgA (<0.0009 g/l). Five of them had a history of anaphylactoid reactions to IVIG. However, four of these five patients tolerated subcutaneous immunoglobulin replacement therapy (SCIG). To identify predisposing factors for anti-IgA antibodies and related anaphylactoid reactions, we analyzed the clinical and immunological phenotype of affected patients. All eight IgG anti-IgA-positive patients lacked IgA(+) B cells in peripheral blood. Moreover, CVID patients with retained class-switched CD27(pos) IgM(neg) IgD(neg) memory B cells (Freiburg classification group II) and total IgA deficiency seem to have an increased risk for developing anti-IgA antibodies. In seven of the eight patients, lymphoproliferation was observed (most prominently nodular lymphatic hyperplasia), two had granulomatous disease, and two showed autoimmune phenomena.
机译：抗IgA抗体呈血清反应阳性的常见可变免疫缺陷病（CVID）患者倾向于静脉注射免疫球蛋白替代疗法（IVIG）发生类过敏反应。在88名CVID患者中，我们鉴定出8例具有IgG抗IgA抗体（9％）。所有八个完全缺乏IgA（<0.0009 g / l）。他们中有五个有对IVIG的类过敏反应史。但是，这五位患者中有四位耐受皮下免疫球蛋白替代疗法（SCIG）。为了确定抗IgA抗体和相关类过敏反应的诱因，我们分析了受影响患者的临床和免疫表型。所有八名IgG抗IgA阳性患者在外周血中均缺乏IgA（+）B细胞。此外，保留类别转换的CD27（pos）IgM（neg）IgD（neg）记忆B细胞（弗莱堡分类组II）和总IgA缺乏的CVID患者似乎产生抗IgA抗体的风险增加。 8例患者中有7例观察到淋巴增生（最明显的是结节性淋巴增生），2例肉芽肿性疾病，2例显示自身免疫现象。

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